To describe the outcome of selective management of hepatic venous outflow obstruction (HVOO), based on its presentation and liver function, we reviewed the records of 49 consecutive patients managed at our institution between 1984 and 1993. Twenty-six patients were managed surgically, 12 nonsurgically, and 11 were not treated. Portosystemic shunts (PSS) were performed in 18 patients (patency 83%). Two patients (11%) died postoperatively, 11 (61%) did well (mean follow-up 6.4 years), three (17%) required subsequent orthotopic liver transplantation, and two (11%) died of late liver failure. PSS remained patent if the preoperative pressure gradient between the portal vein and the infrahepatic inferior vena cava was greater than 10 mm Hg and across the intrahepatic inferior vena cava 18 mm Hg or less. All six orthotopic liver transplantations (three as primary treatment and three after failed PSS) were successful (mean follow-up 4.8 years). Five patients underwent other procedures. Nine (75%) of the 12 nonsurgically treated patients did well (mean follow-up 3.8 years). The most important predictor of successful outcome after PSS or medical management was the degree of liver function. All 11 untreated patients died either of end-stage liver failure (n = 7; 63%) or of severe comorbid disease (n = 4; 37%). In patients with preserved liver function, medical management of HVOO can be successful early in the course of the disease; a late presentation necessitates PSS. Orthotopic liver transplantation should be employed in patients with liver failure and may decrease the high mortality rate of HVOO.
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